作者: Avi L. Friedlich , Ashley I. Bush
DOI: 10.1017/CBO9780511544873.014
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摘要: Introduction The non-infectious neurodegenerative disorders, Alzheimer's disease, and amyotrophic lateral sclerosis are heterogeneous with respect to etiology, neuropathology clinical presentation. Yet, these disorders share a number of features in common suggest some pathogenic events. Each disorder is age related characterized by progressive symmetric degeneration discrete populations neurons. associated biochemical markers oxidative attack, each deposition CuZn metalloprotein affected tissue. Molecular genetic analysis has linked autosomal dominant forms AD, PD, sclerosis, respectively, mutations s-amyloid precursor protein, α-synuclein, superoxide dismutase 1. proteins or its proteolytic products may aggregate tissue during the course disease. In this chapter we summarize current knowledge CNS Cu Zn metabolism normal physiology. Then, focusing on AD ALS, review evidence for pathophysiologic linking physiologic toxic activities protein Protein interactions brain copper zinc At active site many enzymes, participates one-electron transfer reactions. Zn, which electrochemically inert, maintains structural stability proteins. addition essential ubiquitous roles brain-specific functions exist metals.